Repeat renal biopsy in children with severe idiopathic tubulointerstitial nephritis

Idiopathic (primary) tubulointerstitial nephritis (TIN) of childhood is relatively rare. Four children, two with concomitant uveitis, aged 8–14 years, with idiopathic TIN who underwent repeat renal biopsy were retrospectively evaluated. At presentation, all had a significant elevation of the urinary ₂-microglobulin/creatinine ratio (2MG ratio), ranging from 10,100 to 44,550, with increased histological indices of tubulointerstitial scores (TI scores) in excess of 6 points. Three of the children received prednisolone (PSL) therapy following diagnosis, while the remaining child received the therapy 30 months after the first renal biopsy. In the children that received prompt PSL therapy, a rapid decrease in urinary 2MG ratio was observed and the TI scores obtained at a mean interval of 16 months after the first biopsy decreased to less than 5, while preserving renal function. In the remaining child that received delayed PSL therapy, persistent elevations of urinary 2MG ratio and TI scores were observed. He subsequently progressed to chronic renal insufficiency. These clinical findings suggest that persistent elevations of urinary 2MG ratio and TI scores are indicators of progression of renal failure in TIN. For successful treatment, early therapeutic intervention should be deployed in selected patients with severe idiopathic TIN.     

Relationship between cochlear implant outcome and the diameter of the cochlear nerve depicted on MRI

This study aimed to evaluate the relationship between the diameters of the auditory and eighth cranial nerves and improvements in post-implant performance. Twenty prelingually deafened children (aged from 2.0 to 6.0 years) who received the Nucleus 24 cochlear implant participated in this study. All subjects had used their implant for at least 1 year after device connection. The diameters of cochlear and eighth cranial nerves were retrospectively measured on preoperative T2-weighted axial magnetic resonance image (MRI). In 17 of 20 subjects, the cochlear and eighth cranial nerves could be identified on MRI. The mean diameter of the cochlear and eighth cranial nerves were 0.9 +/- 0.2 mm and 1.2 +/- 0.3 mm, respectively. In the remaining three subjects, the cochlear and eighth cranial nerves could not be identified on MRI. These three subjects had significantly lower scores in the Infant-Toddler-Meaningful Auditory Integration Scale (IT-MAIS) than the other 17 subjects at 12 months post-implant. There was no significant correlation among the maximal diameters of the nerves and age, ECAP thresholds and IT-MAIS scores. A sufficient outcome from cochlear implantation can be expected when cochlear and eighth cranial nerves are depicted on MRI, regardless of the nerve diameters.     

Usefulness of weekly administration of paclitaxel for advanced or recurrent gastric cancer

We report herein the efficacy and feasibility of weekly administration of paclitaxel for advanced/recurrent gastric cancer retrospectively. Eleven patients with advanced or recurrent gastric cancer who had received prior chemotherapy were treated with this regimen. Seventy mg of paclitaxel per m2 dissolved in 250 ml 5% glucose was administered by 1-hour intravenous infusion once a week for 3 weeks followed by 1 week rest. To avoid hypersensitivity reactions, the following short premedication was given to all the patients 1 hour before paclitaxel treatment: Dexamethasone 20 mg intravenously (i.v.), diphenhydramine 50 mg i.v., and ranitidine 50 mg i.v. Treatment cycle was 1 to 23 with an average cycle of 5.4. The response rate was 33% (2/6 with measurable lesions), the median time to progression was 104 days, and the median survival time was 160 days. Grade 3 neutropenia occurred in 27.2% of the patients. Weekly paclitaxel may be a promising regimen as a second-line chemotherapy for advanced/recurrent gastric cancer. However, special attention needs to be paid to the neutropenic adverse effect in gastric cancer patients with poor performance status than 2 (greater).     

A case of pseudoaldosteronism, accompanied with hypocalcemia and exaggerated ACTH response

Glycyrrhizic acid (GA) inhibits the activity of 11beta-hydroxysteroid dehydrogenase type 2 in the kidney, with the resulting increase in intrarenal cortisol concentration leading to hypertension and suppression of the renin-aldosterone system. In this paper we describe an interesting case of pseudoaldosteronism, associated with hypocalcemia and an exaggerated ACTH response. A 72-year-old woman was referred to our department for further evaluation of hypokalemia and hypocalcemia. The patient had been taking GA (150 mg/day) for the previous year for treatment of liver damage. Plasma renin activity and aldosterone concentration were both within lower normal limits. Urinary excretion of potassium and calcium was within the upper limit of the normal range and increased with administration of supplements. Plasma ACTH levels increased markedly in response to an intravenous injection of CRH. Cessation of GA and the potassium and calcium supplements on admission, led to a gradual normalization of serum potassium and calcium levels and blood pressure. The hypocalcaemia in our patient was related to decreased tubular reabsorption of calcium as a consequence of renal corticoid excess. It is possible that an increase in the number of CRH receptors in the pituitary following GA treatment caused the exaggerated ACTH response in association with pseudoaldosteronism. The existence of hypocalcemia and an exaggerated ACTH response should be observed carefully when managing pseudoaldosteronism.     

Chromogenic assay for the activity of sphingomyelinase from Bacillus cereus and its application to the enzymatic hydrolysis of lysophospholipids

We developed a convenient chromogenic assay method for the activity of sphingomyelinase (SMase) from Bacillus cereus. SMase reaction was quenched by Zn(2+), and the released phosphocholine was converted into a choline by the action of alkaline phosphatase. After that, the choline was converted into a chromogenic dye by the actions of choline oxidase and peroxidase in the presence of EDTA to trap the added Zn(2+) which could interfere with the choline oxidase/peroxidase reactions. Triton X-100 also was added to the reaction mixture, in order to remove turbidity generated from ceramide which had been produced by the SMase reaction. To test a large number of samples in a short period of time, this assay was performed using 96-well microtiter plates. This method proved to be applicable not only to the measurement of the hydrolysis of sphingomyelin but also to those of lysophosphatidylcholine (lysoPC) and lyso platelet-activating factor by B. cereus SMase. Using this method, the kinetic parameters (K(m) and k(cat)) for B. cereus SMase toward various types of substrates were then determined, and the effect of Triton X-100 on the hydrolysis of lysoPC was examined.     

Vitrectomy with or without arteriovenous adventitial sheathotomy for macular edema associated with branch retinal vein occlusion

PURPOSE: compare the results of vitrectomy with or without arteriovenous (AV) crossing sheathotomy for macular edema associated with branch retinal vein occlusion (BRVO). DESIGN: Interventional case series. METHODS: A retrospective study of 36 eyes with BRVO-associated macular edema. Twenty eyes underwent AV sheathotomy (AS group), and 16 eyes underwent posterior vitreous detachment (PVD group). Best-corrected visual acuity (BCVA), fluorescein angiography (FA), and optical coherence tomography to determine foveal thickness were conducted preoperatively and at 12 months postoperatively. RESULTS: The mean postoperative BCVAs were significantly better in both the AS and the PVD group (P = .008 and P = .001, respectively). Foveal thickness decreased significantly 1 month after surgery in both groups (P = .002 and P = .007) and continued to decrease up to 12 months. The postoperative mean BCVA and improvement of BCVA and foveal thickness were not significantly different for the two groups at any postoperative period. Postoperative FA showed reperfusion of the occluded vein in 10 eyes in the AS group and 2 eyes in the PVD group, and formation of shunt vessels at the AV crossing site or around the macular region in all of the other eyes of both groups. CONCLUSIONS: Both AV sheathotomy and simple PVD significantly reduced macular edema associated with BRVO. However, there was no significant difference in the improvement of macular function following either procedure. Postoperative improvement of retinal circulation by either reperfusion of the occluded vein or collateral vessel formation was found. This accounted for functional and morphologic improvements.     

Co-expression of parathyroid hormone and chromogranin A in secondary hyperparathyroidism: a functional marker for secretory activity of hyperplastic nodules

The relationship between secretion of parathyroid hormone (PTH) and biologic characteristics, including cell proliferation or monoclonality, is not yet fully understood. To evaluate secretory activity of glands or nodules histopathologically, we focused on the co-expression of chromogranin A (CgA) and parathyroid hormone (PTH) in each gland or nodule. A total of 55 glands from 38 patients with normal parathyroid glands, hyperplastic glands (diffuse and nodular) and primary adenomas were compared. Co-expression of PTH and CgA was decreased to 44.4% in diffuse hyperplastic glands, and to 39.6% in 91 hyperplastic nodules, in contrast to normal glands and primary adenomas that showed constant co-expression of PTH and CgA. Immunohistochemical study of PTH showed a coarse granular pattern predominantly in PTH-positive/CgA-positive nodules, and a dot-like pattern mainly in PTH-positive/CgA-negative nodules. Laser scanning microscopy and immunoelectron microscopy confirmed that a dot-like pattern is based on a positive reaction of PTH at the Golgi apparatus. MIB-1 LI was 12.6 +/- 11.6 in PTH-positive/CgA-positive, and 19.3 +/- 27.3 in PTH-positive/CgA-negative nodules. In conclusion, a combination of PTH and CgA could provide more information about the physiologic state of secretory activity of each nodule than does the simple observation of PTH immunoreactivity.